DERMATOPATHOLOGY CASES: Self-Assessment Cases: Editor - Dr Sampurna Roy MD

Digital Images of interesting cases that will include the full spectrum of Dermatopathology, presented in the form of quiz.

The answer of the cases include related links and recent abstracts of articles.

Thursday, June 24, 2010

Answer of Dermatopathology Case 43

Subcutaneous Panniculitis-like T-Cell Lymphoma

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Subcutaneous panniculitis-like T-cell lymphoma: redefinition of diagnostic criteria in the recent World Health Organization-European Organization for Research and Treatment of Cancer classification for cutaneous lymphomas.Arch Pathol Lab Med.2009 Feb;133(2):303-8.
Subcutaneous panniculitis-like T-cell lymphoma is a primary T-cell lymphoma that preferentially involves the subcutaneous tissue. Although subcutaneous panniculitis-like T-cell lymphoma has been recognized as a distinctive entity in the category of peripheral T-cell lymphoma in the World Health Organization classification, its diagnostic criteria has been redefined by the recent World Health Organization-European Organization for Research and Treatment of Cancer classification for primary cutaneous lymphomas. Subcutaneous panniculitis-like T-cell lymphoma is now restricted to primary cutaneous T-cell lymphoma expressing alphabeta T-cell receptor phenotype. These lymphomas are usually CD3(+), CD4(-), CD8(+), and CD56(-), and usually have an indolent clinical course. The clinicopathologic features, differential diagnosis, immunophenotypic characteristics, and molecular features of subcutaneous panniculitis-like T-cell lymphoma are presented in light of the recent World Health Organization-European Organization for Research and Treatment of Cancer classification.

Two cases of subcutaneous panniculitis-like T-cell lymphoma (CD4- CD8+ CD56-)]Ann Dermatol Venereol. 2009 Mar;136(3):264-8. Epub 2008 Dec 6.
BACKGROUND: Subcutaneous T-cell lymphoma is a rare disease and diagnosis is often difficult. We report two cases of subcutaneous T-cell lymphoma simulating panniculitis and initially treated with systemic steroids. PATIENTS AND METHODS: Case No. 1. A 75-year-old woman, otherwise asymptomatic, presented with plaques and nodules of the legs present for 4 months. Histological and immunohistochemical analysis revealed subcutaneous T-cell lymphoma of CD4- CD8+ CD56- phenotype. Improvement was observed with systemic steroids followed by radiotherapy. Case No. 2. A 53-year-old woman presented with a 10-year history of recurrent infiltrated plaques. Lupus panniculitis was evoked but not confirmed. In the context of new panniculitis lesions, histological examination showed a dense lymphocytic infiltrate involving the fat lobules. Lymphocyte immunophenotyping and genotyping led to a diagnosis of subcutaneous T-cell lymphoma of CD4- CD8+ CD56- phenotype. Molecular analysis showed T-cell monoclonality (alpha beta). The lesions improved after few months of systemic corticosteroids. Although new nodular lesions appeared from time to time, further therapy was not deemed necessary. DISCUSSION: A distinction is currently made between two types of subcutaneous T-cell lymphomas and the prognosis and therapeutic consequences differ widely. In our two patients, subcutaneous CD8+ T-cell lymphoma was diagnosed. The clinical course was indolent in both cases. Differential diagnosis with regard to lupus panniculitis can be difficult because of its slow progression. In such cases of low-grade lymphomas, first-line therapy may be limited to systemic corticosteroids.

Subcutaneous panniculitic-like T-cell lymphoma and other primary cutaneous lymphomas with prominent subcutaneous tissue involvement.Dermatol Clin.2008 Oct;26(4):529-40, viii.
The concept of subcutaneous T-cell lymphoma defines a reduced group of primary cutaneous lymphomas characterized morphologically by a prominent or exclusive subcutaneous tissue involvement. Subcutaneous panniculitic-like T-cell lymphoma is a rare subtype of primary cutaneous T-cell lymphoma clinically mimicking panniculitis. The clinical course is usually protracted with recurrent cutaneous lesions but rarely with early extracutaneous dissemination. The clinical, histopathologic, immunophenotypic, and evolutive features of this heterogeneous and rare group of primary cutaneous lymphomas are reviewed.

Subcutaneous panniculitic T-cell lymphoma in a cardiac allograft recipient.J Cutan Pathol. 2005 May;32(5):366-70.
BACKGROUND: Post-transplant lymphoproliferative disorder (PTLD) is the third leading cause of death in heart transplant patients beyond the immediate peri-operative period (Ouseph R, Denny DM, Erbeck KM. J Am Soc Echocardiogr 1998; 11: 758; Armitage JM, Kormos RL, Stuart RS, et al. J Heart Lung Transplant 1991; 10: 877; Swinnen LJ, Mullen M, Carr TJ, et al. Blood 1995; 86: 3333; Ying AJ, Myerowitz D, Marsh WL. Ann Thorac Surg 1997; 64: 1822). The majority of PTLD cases are of B-cell origin whereas T-cell neoplasms have been reported as rare, aggressive, and late complications of solid-organ transplantation (Fatio R, Sutsch G, Mayer K, et al. Transplant Proc 1998; 30: 1118). CASE REPORT: A 50-year-old cardiac allograft heart transplant patient presented with subcutaneous nodules involving his trunk and extremities. RESULTS: Light microscopy revealed features characteristic of subcutaneous panniculitic-like T-cell lymphoma. Immunohistochemical analysis showed expression for CD45RO, TIA-1, and focal CD3 positivity by tumor cells. Flow cytometry performed on a subsequent subcutaneous nodule demonstrated an abnormal T-cell population with expression of CD3, CD8, CD56, and T-cell receptor alpha-beta, and no expression of CD4. T-cell gene rearrangement studies revealed a clonal population of cells with a bi-allelic gene rearrangement. CONCLUSION: We report a case of an unusual subtype of PTLD in a cardiac allograft recipient.

Dermatopathology Case 43

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Case 43

A subcutaneous, tender nodule, on the left leg, of a 45 year of female.

Monday, June 21, 2010

Answer of Dermatopathology Case 42

Lupus Panniculitis (Lupus Profundus)

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Normal subcutaneous fat, necrosis of adipocytes and classification of the panniculitides.Semin Cutan Med Surg.2007 Jun;26(2):66-70.
The panniculitides represent a group of heterogeneous inflammatory diseases that involve the subcutaneous fat. The specific diagnosis of these diseases requires histopathologic study because different panniculitides usually show the same clinical appearance, which consists of erythematous nodules on the lower extremities. However, the histopathologic study of panniculitis is difficult because of an inadequate clinicopathologic correlation and the changing evolutive nature of the lesions. In addition, large scalpel incisional biopsies are required. From histopathologic point of view, all panniculitides are somewhat mixed because the inflammatory infiltrate involves both the septa and lobules. However, nearly always the differential diagnosis between a mostly septal and a mostly lobular panniculitis is straightforward at scanning magnification on the basis of the structures more intensely involved by the inflammatory infiltrate. Mostly septal panniculitides with vasculitis are actually more vasculitis than panniculitis and include superficial thrombophlebitis and cutaneous polyarteritis nodosa. Mostly septal panniculitides with no vasculitis include erythema nodosum, necrobiosis lipoidica, deep morphea, subcutaneous granuloma annulare, rheumatoid nodule, and necrobiotic xanthogranuloma. Mostly lobular panniculitis with vasculitis is only represented by erythema induratum of Bazin. In contrast, mostly lobular panniculitides without vasculitis comprise a large series of disparate disorders, including sclerosing panniculitis, calciphylaxis, sclerema neonatorum, subcutaneous fat necrosis of the newborn, poststeroid panniculitis, lupus erythematosus profundus, pancreatic panniculitis, alpha(1)-antitrypsin deficiency panniculitis, subcutaneous Sweet syndrome, infective panniculitis, factitial panniculitis, lipodystrophy, traumatic panniculitis, subcutaneous sarcoidosis, and sclerosing postirradiation panniculitis. Finally, some cutaneous lymphomas may simulate panniculitis, both from clinical and histopathologic points of view and, for that reason, they will be included in this review, although they are not inflammatory processes, but authentic lymphocytic neoplasms involving subcutaneous tissue.

Lupus erythematosus panniculitis: clinicopathological, immunophenotypic, and molecular studies. Am J Dermatopathol.2010 Feb;32(1):24-30.
Lupus erythematosus panniculitis (LEP) is an inflammatory disorder of the subcutaneous fat in patients with lupus erythematosus (LE). It is a rare variant of the disease, which occurs approximately in 1%-3% of patients with cutaneous LE. The purpose of this study was to investigate the clinical, histopathologic, immunophenotypical, and molecular profiles of LEP. We performed a retrospective study of 19 biopsy specimens from 17 patients with LEP. We reviewed their clinical data and reexamined the histopathology. Immunophenotyping and molecular studies were done using sections from paraffin-embedded formalin-fixed tissue. The most common clinical manifestation was a depressed patch on upper arm. Patients showed good response to variable treatment modalities, but, generally, relapse of panniculitis was noted when treatment was discontinued. Histopathologically, most specimens revealed lymphoplasmacytic lobular panniculitis with epidermal and dermal changes of LE, hyaline fat necrosis, and lymphoid follicles. Immunohistochemistry showed a mixture of T and B cells in dermis and subcutis with a slight preponderance of T cell. Although the polymerase chain reaction analysis of the T-cell receptor-gamma gene rearrangement showed a polyclonal smear in 89.5% of cases, a small portion of specimens demonstrated monoclonality. LEP is a chronic recurrent disease with characteristic features. Its diagnosis is often challenging, and a precise diagnosis is achievable only upon elaborate clinicopathologic correlation and integrated interpretation of all diagnostic criteria.

Lupus profundus, indeterminate lymphocytic lobular panniculitis and subcutaneous T-cell lymphoma: a spectrum of subcuticular T-cell lymphoid dyscrasia.J Cutan Pathol. 2001 May;28(5):235-47.
INTRODUCTION: The diagnosis and classification of lymphocytic lobular panniculitis (LLP) has historically proven to be a difficult challenge. We encountered 32 cases of primary LLP which could be categorized as: 1) lupus erythematosus profundus (LEP) (19 patients); 2) an indeterminate group termed indeterminate lymphocytic lobular panniculitis (ILLP) (6 patients); and 3) subcutaneous T-cell lymphoma (SCTCL) (7 patients). OBJECTIVE: We attempted to better define the subtypes of LLP by morphologic, phenotypic and genotypic features and to correlate those features to clinical presentation and outcome. METHOD: Skin biopsy material was studied by conventional light microscopy, through immunophenotyping performed on sections from paraffin-embedded, formalin-fixed tissue and in some cases on sections of tissue frozen after receipt in physiological (Michel's) medium, and by polymerase chain reaction single-stranded conformational polymorphism analysis to assess for clonality of T-lymphocytes. Clinical features were correlated to histologic, phenotypic, and genotypic analyses. RESULTS: Patients with LEP had a prior diagnosis of LE or overlying skin changes which light microscopically were characteristic of LE. Patients with ILLP had no concurrent or prior history of LE, no systemic symptoms or cytopenias, and a clinical course not suggestive of lymphoma. Cases of SCTCL showed hemophagocytic syndrome and/or lesional progression with demise attributable to the disease. Lesions in all groups showed proximal extremity predilection. Females predominated in the LEP group. The average age of onset was 38, 40 and 55 years in the LEP, ILLP and SCTCL groups, respectively. Cytopenia was seen in 4 LEP patients; 1 also developed fever. In LEP and ILLP, lesions resolved with hydroxychloroquine and/or steroid therapy, with recurrences following cessation of therapy. In the SCTCL group 4 developed hemophagocytic syndrome, 4 died within 2 years of diagnosis, and 3 went into remission following chemotherapy. The LEP and SCTCL groups manifested histological similarities: dense perieccrine and lobular lymphocytic infiltration, lymphoid atypia, histiocytes with ingested debris, eosinophilic necrosis of the fat lobule and thrombosis. The atypical lymphocytes although pleomorphic did not have a cerebriform morphology. The infiltrate in ILLP had a similar cytomorphology and distribution with variable angioinvasion which in all save one case was of lesser intensity and was not associated with significant fat necrosis or vasculitis. Germinal centers, dermal/subcuticular mucin deposition and an atrophying interface dermatitis with hyperkeratosis and follicular plugging were largely confined to the LEP group. Erythrophagocytosis, characteristic of SCTCL, usually indicated a supervening subcuticular lymphoid dyscrasia when encountered in ILLP and LEP. SCTCL showed a selective loss of CD5 expression with or without diminution in CD7 and monoclonal CD3 expression. Of 4 cases studied, 3 showed a CD8 dominant infiltrate while 2 others exhibited CD56 and CD30 positivity, respectively. All cases of SCTCL with amplifiable DNA showed T-cell clonality. Similar molecular and phenotypic features indicative of subcuticular lymphoid dyscrasia were encountered in cases of LEP and ILLP including a reduction in CD5, CD7, and/or monoclonal CD3 expression, a preponderance of CD8 lymphocytes within the subcutaneous fat and T-cell clonality. These cases showed lymphoid atypia with variable erythrophagocytosis. Cases of phenotypically abnormal and/or clonal LEP showed one or more of local destruction, lesional size progression, fever, and cytopenias, but lesions responded to hydroxychloroquine and/or prednisone therapy and death attributable to panniculitis could not be documented. Cases that were phenotypically normal and without clonality had none of the aforesaid atypical clinical features. CONCLUSION: Lymphoid atypia, erythrophagocytosis, loss of certain pan T-cell markers, a reduced CD4/8 ratio and TCR rearrangement define subcuticular T-cell lymphoid dyscrasia, including a subset of LEP and ILLP. The subcuticular lymphoid infiltrates represent a spectrum of histologic, immunophenotypic, and molecular abnormalities which range from those which are clearly benign to those which are clearly neoplastic, and also encompasses those cases which defy precise classification into the two aforesaid

Sunday, June 20, 2010

Dermatopathology Case 42

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Case 42

A 50 year old female, with multiple large indurated plaques and nodules, on the upper extremities.